Connective tissue nevus D23.L

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 26.05.2024

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Synonym(s)

Cobblestone nevus; Collagen nevus; collagenous nevus lumbosacralis; Collagen rich connective tissue nevus; connective tissue nevus; Lumbosacral connective tissue nevus; Lumbosacral nevus; Paving stone nevus; Shagreen stain

Definition
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Congenital malformation of the corial connective tissue, usually localized in the sacral region. Occurs either in isolation or as a partial symptom of Pringle-Bourneville phacomatosis or Buschke-Ollendorf syndrome. No malignant development.

Classification
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Solitary collagen nevus (usually spontaneous occurrence)

Large nodular disseminated connective tissue nevus (familial occurrence-primarily localized on the trunk)

Multiple occurring connective tissue nevi (familial cutaneous collagenoma; OMIM 115250)

Localization
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The solitary form is almost exclusively located in the lumbosacral region. In principle, however, every bodily cell can be affected.

Clinical features
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Multiple, skin-coloured or whitish, symptomless, grouped standing or confluent to larger plaques (cobblestone-like aspect), flat raised nodules, plaques or knots of 0.2-5.0 cm in diameter.

Histology
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Increased, densely packed collagenous connective tissue with clumpy collagen fibres. Reduced and altered elastic fibre texture, sometimes intertwined like a band. The changes are usually discreet and can only be assessed in comparison to the normal dermis.

Diagnosis
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The clinical picture in typical localization is diagnostic.

Differential diagnosis
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Connective tissue nevi of different genesis.

Therapy
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No therapy necessary. Excision if necessary.

Literature
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  1. Cesinaro AM (2003) "Connective tissue nevus" and a serendipitous S-100 discovery. Am J Dermatopathol 25: 86-87
  2. el Fekih N et al. (1993) A case for diagnosis: connective tissue nevi of the skin (hamartoma)] Ann Dermatol Venereol 120: 639-641
  3. Martelli H et al. (1994) Congenital soft tissue dysplasias: a morphological and biochemical study. Pediatr Pathol 14: 873-894
  4. Roth AM, Keltner JL (1993) Linear nevus sebaceous syndrome. J Clin Neuroophthalmol 13: 44-49
  5. Yang Q et al (2022) Connective tissue nevus-Isolated collagenoma. Skin Res Technol 28:880-881.
  6. Zelger B (2002) Connective tissue tumors. Recent Results Cancer Res 160: 343-350

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 26.05.2024